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    Pemphigus Vegetans of Neumann: A Case Report of A Rare Variant of Pemphigus Vulgaris

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    IJAR JOURNAL
    Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by periorificial papillomatous vegetations. We present the case of a 77-year-old female patient with a history of type 2 diabetes and hypertension who presented with painful verrucous lesions on her tongue. The examination revealed a cerebriform tongue with small, hard, confluent verrucous swellings, which is a well-known sign seen in pemphigus vegetans. A biopsy revealed acantholysis and absence of keratinocyte necrosis, l

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    Pemphigus Vegetans of Neumann: A Case Report of A Rare Variant of Pemphigus Vulgaris

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    IJAR JOURNAL
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    Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by periorificial papillomatous vegetations. We present the case of a 77-year-old female patient with a history of type 2 diabetes and hypertension who presented with painful verrucous lesions on her tongue. The examination revealed a cerebriform tongue with small, hard, confluent verrucous swellings, which is a well-known sign seen in pemphigus vegetans. A biopsy revealed acantholysis and absence of keratinocyte necrosis, l

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    PEMPHIGUS VEGETANS OF NEUMANN: A CASE REPORT OF A RARE VARIANT OF PEMPHIGUS VULGARIS

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    Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by periorificial papillomatous vegetations. We present the case of a 77-year-old female patient with a history of type 2 diabetes and hypertension who presented with painful verrucous lesions on her tongue. The examination revealed a cerebriform tongue with small, hard, confluent verrucous swellings, which is a well-known sign seen in pemphigus vegetans. A biopsy revealed acantholysis and absence of keratinocyte necrosis, l

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    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    19 views3 pages

    Pemphigus Vegetans of Neumann: A Case Report of A Rare Variant of Pemphigus Vulgaris

    Uploaded by

    IJAR JOURNAL
    Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by periorificial papillomatous vegetations. We present the case of a 77-year-old female patient with a history of type 2 diabetes and hypertension who presented with painful verrucous lesions on her tongue. The examination revealed a cerebriform tongue with small, hard, confluent verrucous swellings, which is a well-known sign seen in pemphigus vegetans. A biopsy revealed acantholysis and absence of keratinocyte necrosis, l

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    © All Rights Reserved
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    of 3

    ISSN: 2320-5407 Int. J. Adv. Res.

    11(04), 982-984

    Journal Homepage: - www.journalijar.com

    Article DOI: 10.21474/IJAR01/16750


    DOI URL: http://dx.doi.org/10.21474/IJAR01/16750

    RESEARCH ARTICLE
    PEMPHIGUS VEGETANS OF NEUMANN: A CASE REPORT OF A RARE VARIANT OF PEMPHIGUS
    VULGARIS

    Dr. Sektaoui Soukaina, Dr. Mehsas Zoubida, Dr. Asermouh Marwa, Pr. Meziane Meriem, Pr. Ismaili Nadia,
    Pr. Benzekri Laila and Pr. Senouci Karima
    Department of Dermatology & Venereology, Ibn Sina University Hospital, Faculty of Medicine and Pharmacy of
    Rabat, Mohamed V University, Rabat, Morocco.
    ……………………………………………………………………………………………………....
    Manuscript Info Abstract
    ……………………. ………………………………………………………………
    Manuscript History Pemphigus vegetans is a rare variant of pemphigus vulgaris
    Received: 28 February 2023 characterized by periorificial papillomatous vegetations. We present the
    Final Accepted: 31 March 2023 case of a 77-year-old female patient with a history of type 2 diabetes
    Published: April 2023 and hypertension who presented with painful verrucous lesions on her
    tongue. The examination revealed a cerebriform tongue with small,
    Key words:-
    Pemphigus Vegetans, Pemphigus hard, confluent verrucous swellings, which is a well-known sign seen
    Vulgaris, Cerebriform Tongue, in pemphigus vegetans. A biopsy revealed acantholysis and absence of
    Neumann, Acantholysis keratinocyte necrosis, leading to the diagnosis of Neumann-type
    pemphigus vegetans. The patient was prescribed prednisone and
    trichloroacetic acid, which resulted in successful treatment.
    Management of pemphigus vegetans should be individualized based on
    the patient's clinical presentation and response to treatment.

    Copy Right, IJAR, 2023,. All rights reserved.


    ……………………………………………………………………………………………………....
    Introduction:-
    Pemphigus vulgaris is a rare, autoimmune disorder of the skin and mucous membranes that is characterized by the
    production of autoantibodies, particularly IgG antibodies, against desmoglein (1). There are two subtypes : The
    Neumann and the Hallopeau type (2). Here we present a case of Neumann-type pemphigus vegetans in a 77-year-old
    female patientwith a history of type 2 diabetes and hypertension. The patient presented with painful verrucous
    lesions on her tongue, which was characterized by a cerebriform appearance.

    Case Report :
    We present the case of a 77-year-old female patient, who was being treated for type 2 diabetes and hypertension, and
    presented to our clinic with painful verrucous lesions on her tongue. She reported experiencing these lesions
    intermittently for the past 15 years. During the patient interview, we discovered that she had been taking
    corticosteroids irregularly.Upon examination of the oral mucosa, we observed a cerebriform tongue (Figure 1),
    depapillated, pseudo-leukoplakic with heterogeneous coloration. The center of the tongue was covered with small,
    hard, confluent verrucous swellings.This cerebriform appearance is a well-known sign seen in pemphigus vegetans.
    No anomalies were detected during the rest of the dermatological examination.A tongue biopsy revealed a
    malpighian layer with a deep suprabasal cleavage, acantholysis, and absence of keratinocyte necrosis. The basal
    cells had a "tombstone" appearance. Additionally, the patient had elevated anti-intercellular substance antibodies at a
    titer of 1280. All these findings led us to conclude the diagnosis of pemphigus vegetansof Neumann.To treat this
    pathology, the patient was prescribed prednisone at a dosage of 2 mg/kg/day, with a gradual reduction in dosage,
    and 30% trichloroacetic acid applied every two weeks. The lesions disappeared as a result of the treatment.

    Corresponding Author:- Dr. Sektaoui Soukaina 982


    Address:- Department of Dermatology & Venereology, Ibn Sina University Hospital, Faculty
    of Medicine and Pharmacy of Rabat, Mohamed V University, Rabat, Morocco.
    ISSN: 2320-5407 Int. J. Adv. Res. 11(04), 982-984

    Figures

    Figure 1:- Clinical image showing the cerebriform tongue, a well-known sign in pemphigus vegetans, characterized
    by a typical pattern of sulci and gyri over the dorsum of the tongue, resembling the appearance of the brain's surface.

    Discussion:-
    Pemphigus vulgaris is a rare, autoimmune disorder of the skin and mucous membranes that is characterized by the
    production of autoantibodies, particularly IgG antibodies, against desmoglein, a transmembrane protein that is
    essential for maintaining intercellular adhesion in the skin and mucous membranes. These autoantibodies disrupt the
    normal function of desmoglein, leading to a process known as acantholysis, where the keratinocytes in the skin and
    mucous membranes separate from each other, resulting in the formation of blisters and erosions (1). Pemphigus
    vegetans, which is a rare variant of pemphigus vulgaris, was first described by Neumann in 1876. He can be
    differentiated into two subtypes based on their clinical occurrence, course, response to treatment, and prognosis :
    The Neumann type, which is characterized by periorificial papillomatous vegetations, and the Hallopeau type, which
    is characterized by pustular lesions that evolve into vegetations and preferentially affect the intertriginous areas.
    This subtype typically has a benign course with few relapses (2, 3).The cerebriform tongue, which is observed in the
    clinical appearance of our patient (Figure 1), is a well-known sign in pemphigus vegetans, described as the
    "Premalatha sign." This distinctive feature is characterized by a typical pattern of sulci and gyri over the dorsum of
    the tongue, resembling the appearance of the brain's surface. He can be used as a clinical clue for its diagnosis
    pemphigus vegetans, especially when combined with other clinical features and histopathological findings (4).
    Based on the physical examination, we diagnosed our patient with Neumann-type pemphigus vegetans, and a skin
    biopsy was performed to confirm the diagnosis. The treatment of pemphigus vegetans is similar to pemphigus
    vulgaris and typically involves the use of systemic corticosteroids. However, response to oral corticosteroid
    treatment may be variable, and disease remission may not always be achieved (5). In our case, we opted to initiate

    983
    ISSN: 2320-5407 Int. J. Adv. Res. 11(04), 982-984

    treatment with oral corticosteroids and switch to a systemic approach, if necessary, but this was not required, as our
    patient responded well to the initial therapy. In addition to corticosteroids, the use of immunosuppressants may be
    beneficial in improving disease remission rates and reducing the need for long-term corticosteroid therapy (5, 6). In
    some cases, other medications such as dapsone and retinoids have also been used successfully as adjunctive therapy
    (7).It is important to note that the management of pemphigus vegetans should be individualized based on the
    patient's clinical presentation, disease severity, and response to treatment.

    Conclusion:-
    In conclusion, we present a case of a 77-year-old female patient with painful verrucous lesions on her tongue, who
    was diagnosed with Neumann-type pemphigus vegetans based on clinical, histopathological, and laboratory
    findings. The patient responded well to oral corticosteroid treatment and the application of 30% trichloroacetic acid.
    This case highlights the importance of recognizing the cerebriform tongue appearance as a clinical clue for the
    diagnosis of pemphigus vegetans and individualizing the management of the disease based on the patient's clinical
    presentation, disease severity, and response to treatment. Further research is needed to identify alternative treatment
    options for patients who do not respond to conventional therapies.

    Acknowledgements:-
    Sektaoui Soukaina, Mehsas Zoubida, AsermouhMarwa participated in the research design, the writing of the paper.
    Pr Meziane Meriem, Pr Ismaili Nadia, Pr Benzekri Laila and Pr Senouci Karima participated in the research design

    References:-
    1. Dhamija A, D'souza P, Meherda A, Kothiwala RK. Pemphigus vegetans: An unusual
    presentation. IndianDermatol Online J. 2012;3:193–5
    2. Bystryn JC, Rudolph JL. Pemphigus. Lancet. 2005;366:61-73
    3. Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin. 1993;11:429-52
    4. Rebello MS, Ramesh BM, Sukumar D, Alapatt GF. Cerebriform Cutaneous Lesions in Pemphigus
    Vegetans. Indian J Dermatol. 2016;61(2):206-208.
    5. Ichimiya M, Yamamoto K, Muto M. Successful treatment of pemphigus vegetans by addition of etretinate to
    systemic steroids. Clin ExpDermatol. 1998;23:178-80.
    6. Werth VP, Fivenson D, Pandya AG, et al. Multicenter randomized, double-blind, placebocontrolled, clinical
    trial of dapsone as a glucocorticoid-sparing agent in maintenancephase pemphigus vulgaris. Arch Dermatol.
    2008;144:25-32.
    7. Lin MH, Hsu CK, Lee JY. Successful treatment of recalcitrant pemphigus vulgaris and pemphigus vegetans
    with etanercept and carbon dioxide laser. Arch Dermatol. 2005;141:680-2.

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